Fig. 4.

DM1-related pathogenic phenotypes in adult SC mice carrying quadruple mutations. a EMG analysis indicates typical waxing-waning myotonia in QKO SC mice (WT SC mice, n = 3; DSMD-QKO SC mice, n = 5, 4-6-month old). b Forelimb grip strength test (WT SC mice, n = 5; DSMD-QKO SC mice, n = 7, 4-6-month old). Unpaired Student’s t-test, **P < 0.01. c TA CSA analysis (n = 3 per group, 4-6-month old). d Nuclear clump analysis of TA muscle (WT SC mice, n = 6; DSMD-QKO SC mice, n = 3, 4-6-month old). Unpaired Student’s t-test, **P < 0.01. e The percentage of myofibers containing centrally located nuclei in TA muscle of QKO and WT SC mice (P30) (n = 3 per group). Unpaired Student’s t-test, *P < 0.05. f Representative images of ATPase staining and fiber type analysis (n = 4 per group, 4-6-month old). Unpaired Student’s t-test, *P < 0.05, **P < 0.01, ***P < 0.001. Scale bars, 100 μm. g Dust-like opacities in eyes of DSMD-QKO SC mice (3/7, 4-6-month old). h The percentage of myofibers containing centrally located nuclei in TA muscles of QKO and WT F1 mice (n = 5 per group, P0). Unpaired Student’s t-test, ***P < 0.001